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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2-3
pubmed:dateCreated
1986-9-17
pubmed:abstractText
Twenty-five muscle biopsies (18 from the left biceps and 7 from the left quadriceps) of 25 patients suffering from myotonic dystrophy (MyD) were studied, 13 of which showed "ragged-red" fibres (RRFs); all the RRFs, which were type I fibres, were found in biceps muscles, while none of the quadriceps muscles showed RRFs. The incidence of RRFs varied from 0.5% to 20.0% (average 4.2%). On electron microscopy, RRFs contained enlarged mitochondria, usually in subsarcolemmal clusters, including dense granular matrix materials, concentrically whired membranous cristae, and paracrystalline inclusions, consistent with those of previously reported cases of mitochondrial myopathy, suggesting that RRFs observed in biopsies from patients with MyD are due to abnormal mitochondria. The biopsy findings indicative of MyD including pyknotic nuclear clumps, moth-eaten fibres, ring fibres, type I fibre atrophy, and type I fibre predominance, were much more common findings in biceps muscles than quadriceps muscles, and in biopsies with RRFs than those without RRFs. From our observations, it is possible that RRFs in biopsied muscles from patients with MyD are not incidental observations but are intimately associated with the pathogenesis of this disorder, and that RRFs may be a special form of pathological reaction in which accumulation of abnormal mitochondria occurs.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
0022-510X
pubmed:author
pubmed:issnType
Print
pubmed:volume
74
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
247-55
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1986
pubmed:articleTitle
"Ragged-red" fibres in myotonic dystrophy.
pubmed:publicationType
Journal Article